AOCCN2017

講演情報

Poster Presentation

[P1-1~141] Poster Presentation 1

2017年5月11日(木) 09:30 〜 16:00 Poster Room A (1F Navis A・B・C)

[P1-119] Alopecia as the initial manifestation of neuromyelitis optica spectrum disorder in a 7-year-old girl

Xiaofan Yang (Department of Pediatrics, Qilu Hospital of Shandong University, Jinan, China)

[OBJECTIVE] Neuromyelitis optica spectrum disorder (NMOSD) in children with alopecia is a rarely reported occurrence. Here, we present a 7-year old girl with longitudinal myelitis who had a 3-year history of alopecia and a positive antibody to aquaporin-4. [METHODS] A combination of clinical characterization and follow-up, MRI scan and laboratory investigations. [RESULTS] We report the case of a 7-year old girl with a 3-year history of recurrent alopecia and mild mental retardation, who presented with limb weakness and trembling, unsteady walking and urinary retention after upper respiratory infection for 5 days. White blood cell count was normal in the cerebral spinal fluid (CSF), as well as negative results for virus and bacteria tests. Myelin basic protein (MBP) was elevated in both serum and cerebral spinal fluid. Brain and spinal cord MRI showed extensive swelling in the spinal cord. Multiple bilateral and asymmetrical lesions in subcortical white matter and longitudinal abnormal signals in the spinal cord were detected with no obvious enhancement. Testing for aquaporin-4 antibody was positive in the serum. And thus the diagnosis of NMOSD was made. Intravenous immunoglobulin and high-dose intravenous methylprednisolone and a taper of oral methylprednisolone were given, leading to a significant clinical improvement. During the taper the patient's condition deteriorated twice, and received another two cycle of steroid therapy, which lead to a stable improvement. During a 6-month follow-up, her hair grew out and got both clinical and radiological improvement. [CONCLUSIONS] This case suggests autoimmune conditions such as alopecia might co-existing with NMOSD.